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Irradiated Blm-deficient mice are a highly tumor prone model for analysis of a broad spectrum of hematologic malignancies.
Warren, Madhuri Chung, Yeun-Jun Howat, William J Harrison, Hannah McGinnis, Ralph Hao, Xingpei McCafferty, John Fredrickson, Torgny N Bradley, Allan Morse, Herbert C
Warren, Madhuri
Chung, Yeun-Jun
Howat, William J
Harrison, Hannah
McGinnis, Ralph
Hao, Xingpei
McCafferty, John
Fredrickson, Torgny N
Bradley, Allan
Morse, Herbert C
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Abstract
Mutations in the BLM gene cause human Bloom syndrome (BS), an autosomal recessive disorder of growth retardation, immunodeficiency and cancer predisposition. Homozygous null Blm(m3/m3) mice are cancer prone with a 5-fold increased risk of cancer compared with Blm(m3/+) and Blm(+/+) mice. Irradiation of Blm(m3/m3) mice increased the risk to 28-fold. Tumors occurred mainly in the hematopoietic system and were similar to those in BS based on detailed histologic and immunohistochemical analyses. Irradiated Blm-deficient mice thus provide a novel model for understanding accelerated malignancies in BS and a new platform for investigating the molecular basis for a wide range of hematopoietic neoplasms.
Description
Date
2010-02
Publisher
Collections
Keywords
Haematologic Cancer
Type
Article
Citation
Irradiated Blm-deficient mice are a highly tumor prone model for analysis of a broad spectrum of hematologic malignancies. 2010, 34 (2):210-20 Leuk Res